Thyroid stimulating hormone (TSH)-secreting pituitary adenomas are rare tumors of the pituitary gland and represent 1~2% of all pituitary adenomas. A TSH-secreting pituitary adenoma shows as a normal or elevated thyrotropin level in a hyperthyroid patient. We present a 32-year-old woman who was diagnosed with a TSH-secreting pituitary microadenoma. She had a high free T4, with a normal TSH and alpha-subunit. Bilateral inferior petrosal sinus sampling (IPSS) was done to confirm the alpha-subunit secreting adenoma, and the concentration of the alpha-subunit was high on the tumor side. The pituitary microadenoma was removed, and her TSH and free T4 levels decreased to normal. IPSS may help give an accurate diagnosis in the patient with a normal alpha-subunit.
Adenoma ; Adult ; Female ; Humans ; Petrosal Sinus Sampling ; Pituitary Gland ; Pituitary Neoplasms ; Thyrotropin

BACKGROUND: While inferior petrosal sinus sampling (IPSS) correctly diagnoses pituitary dependent Cushings syndrome if a significant ratio of plasma ACTH between the IPS and the peripheral blood is demonstrated, little has been said about the significance of a negative ratio in Cushings disease (e.g. false negative result). We evaluated the results of IPSS with Cushings disease, and compared them with imaging findings through transsphenoidal surgery. METHODS: 29 patients with Cushings disease underwent transsphenoidal examination of the pituitary gland from 1989 to 1998 at Seoul National University Hospital were evaluated. We compared the results of IPSS and imaging findings with sellar CT or dynamic MRI. The ratio of the ACTH concentrations at the IPS and in the peripheral blood (IPS:P ratio) and the ratio of the ACTH concentrations between the IPSs (interpetrosal ratio) were calculated before and after CRH infusion. RESULTS: With IPSS the diagnosis of Cushings disease was possible in 90% (26/29), and 3 cases in whom IPSS did not show significant IPS:P ACTH ratio were confirmed to be Cushings disease through hemihypophysectomy of lesion suspected by sellar dynamic MRI and achieved remission after operation (e.g. false negative result of diagnosis for Cushings disease by IPSS). However, accurate localization of microadenoma was achieved in only 59% (17/29). Imaging study detected microadenoma in 76% (22/29) and correctly localized in 66% (19/29). Both IPSS and imaging study precisely localized the pituitary microadenoma in 10 (34.5%) cases of 29 cases and a discrepancy between two studies existed in 7 (24.1%) cases in which the imaging study correctly localized microadenoma in 6 cases and IPSS in 1 case. CONCLUSION: Only when a significant IPS:P ACTH ratio is present can Cushings disease be established by IPSS. The absence of a significant IPS:P ACTH ratio does not necessarily imply ectopic secretion of ACfH, nor does it exclude Cushings disease. The results of lateralization by IPSS do not remove the need for transsphenoidal examination of the sella turcica because false negative result can be. IPSS and radiologic study should be complementary used in diagnosis of Cushings disease and localization of microadenoma, as IPSS can be used when sellar imaging study failed to visualize the lesion and hemihypophysectomy of suspected lesion by imaging study can be considered when IPSS did not show significant ratio of ACTH.
Adrenocorticotropic Hormone ; Diagnosis* ; Humans ; Magnetic Resonance Imaging ; Petrosal Sinus Sampling* ; Pituitary Gland ; Plasma ; Sella Turcica ; Seoul

Empty Sella Syndrome ; diagnosis ; Female ; Humans ; Middle Aged ; Petrosal Sinus Sampling ; methods ; Pituitary Neoplasms ; diagnosis

PURPOSE: Bilateral inferior petrosal sinus sampling (IPSS) is a direct method of distinguishing between pituitary and ectopic ACTH secretion. We present unilateral femoral route technique avoiding bilateral femoral venous puncture using two 4F catheters into both sides of IPSS in these obese patients. MATERIALS AND METHODS: Unilateral femoral puncture using 9F guiding catheter allowed two 4F catheters which can be introduced in each side of inferior petrosal sinus. To reduce bleeding in the gap between 2 catheters at the diaphragm of the 9F femoral sheath, we introduced a short guidewire provided along with femoral sheath. After removing the 9F sheath after procedure, we applied manual compression as usual. We evaluated any technical difficulty and other complications including the presence of hematoma at the puncture site 1 day and 30 days later. RESULTS: Bilateral IPSS by using two 4F catheters in both inferior petrosal sinuses was possible via unilateral femoral route via 9F sheath. There was no technical difficulty introducing 4F catheters into each IPS of both sides. After removing 9F femoral sheath, there was no other complication and no hematoma at the puncture site at 1 day and 30 days later. CONCLUSION: Unilateral femoral venous approach with a 9-French sheath can be used in IPSS. This technique allowed to pass two 4F catheters for IPSS at both sides and could avoid unnecessary bilateral femoral puncture in these obese patients without any hematoma formation after the procedure.
Adrenocorticotropic Hormone ; Catheters ; Diaphragm ; Hematoma ; Hemorrhage ; Humans ; Petrosal Sinus Sampling ; Punctures

Bilateral inferior petrosal sinus sampling(IPSS) is known to be useful for the differential diagnosis of ACTH-dependent Cushing's syndrome and for the preoperative localization of pituitary microadenomas.To evaluate the usefulness of IPSS, we performed the procedure in the 17 cases of ACTH-dependent Cushing's syndrome including 2 cases of macroadenomas, 6 of them with CRH stimulation test. A inferior petrosal sinus-to-peripheral ACTH ratio of 2:1 or greater(3:1 after CRH stimulation) indicates a pituitary source of ACTH hypersecretion, and a ratio of the ACTH level in one inferior petrosal sinus to the ACTH level in the other of 1:4 or greater lateralizes the microadenomas to that half of the pituitary gland.With these criteria, we diagnosed Cushing's disease in 15 of 17 cases of ACTH-dependent Cushing's syndrome, and localized the lesion in 8 of 13 cases of microadenoma.In conclusion, IPSS with CRH stimulation has high diagnostic accuracy in the differential diagnosis of ACTH-dependent Cushing's syndrome, but still has the limitations on the localization of microadenoma.
Adrenocorticotropic Hormone ; Clinical Study ; Cushing Syndrome ; Diagnosis, Differential ; Petrosal Sinus Sampling

Background: Inferior petrosal sinus sampling(IPSS) is known to be useful for the differential diagnosis of ACTH-dependent Cushings syndrome and for the preoperative lateralization of pituitary microadenoma. We tried to analyze the relative value of IPSS in localization of microadenoma as compared with sella imaging study including computerized tomogram(CT) or magnet resonance imaging(MRI) in Cushings disease. Methods: We reviewed the clinical records of 21 patients with Cushings disease who underwent IPSS and the radiologic study such as sella CT or sella MRI preoperatively followed by transsphenoidal microsurgery. By pathologic examination including immunohistochemistry and postoperative clinical and biochemical evaluation we confirmed the diagnosis of Cushings disease due to pituitary microadenoma in all 21 cases. Results: Sella CT or sella MRI detected microadenoma in 57.1% of cases( =12/21), while recently available dynamic MRI did so in 7 out of S cases. With IPSS the diagnosis of Cushings disease was possible in 90.5% of cases(= 19/21), but accurate lateralization of microadenoma was achieved in only 63.2% of cases( =12/19). IPSS precisely localized the pituitary microadenoma in 6 out of 9 cases whose lesion were not detected by the radiologic study. Of 7 cases in which IPSS failed to localize microadenoma, the radiologic study detected the lesion in 6 cases. Of 5 cases in which IPSS and the radiologic study showed a discrepancy in location of microadenoma, the radiologic study correctly localizaed the lesion in 4 cases and IPSS did so in one case. Conclusion: IPSS is not more reliable than sella imaging study for preoperative localization of microadenoma in Cushings disease. However it might have a complementary role, especially when sella imaging study failed to visualize the lesion.
Diagnosis ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Microsurgery ; Petrosal Sinus Sampling

Cushing's syndrome results from prolonged exposure to high levels of glucocorticoid hormones. ACTH-dependent Cushing's syndrome accounts for about 85% of endogenous causes. Autonomous pituitary ACTH secretion, called Cushing's disease, is responsible for 80% of these causes, whereas ectopic ACTH secretion is responsible for 20% of them. Appropriate therapy of patients with Cushing's syndrome depends on accurate diagnosis and classification of the disorder. In addition to the history and clinical evaluation, the laboratory evaluation of a patient with Cushingoid appearance is necessary to establish the diagnosis and determine the cause of hypercortisolism. A major problem in the differential diagnosis of ACTH-dependent Cushing's syndrome is distinguishing Cushing's disease from the ectopic ACTH syndrome. Both entities can have similar clinical and laboratory features. In addition, both pituitary microadenoma and ectopic ACTH-secreting tumors may be radiologically occult. Bilateral inferior petrosal sinus and peripheral vein catheterization with simultaneous collection of samples for measurement of ACTH is one of the most specific tests available to localize the source of ACTH production. We report two cases of Cushing's syndrome diagnosed by inferior petrosal sinus sampling associated with normal or low ACTH levels on routine basal hormone assay.
ACTH Syndrome, Ectopic ; Adrenocorticotropic Hormone* ; Catheterization ; Catheters ; Classification ; Cushing Syndrome* ; Diagnosis ; Diagnosis, Differential ; Humans ; Petrosal Sinus Sampling ; Plasma* ; Veins

Esthesioneuroblastoma as a source of ectopic Cushing's syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. ⁶⁸Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Combined transcranial and endoscopic endonasal approach surgery was performed, and a diagnosis of esthesioneuroblastoma was given.
Cushing Syndrome ; Diagnosis ; Esthesioneuroblastoma, Olfactory ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neuroimaging ; Petrosal Sinus Sampling ; Positron-Emission Tomography

Inferior petrosal sinus sampling (IPSS) plays an important role in the assessment of patients with ACTH-dependent Cushing's syndrome. IPSS has been described as an innocuous, essentially risk-free procedure. Neurological complications associated with IPSS are rare. Previously reported neurological complications include brain stem infraction, pontine hemorrhage and subarachnoid hemorrhage. A 26-year-old woman was admitted to the hospital because of a moon face and a buffalo hump. A MRI showed a suspicious, but uncertain, area on the left side of the sella turcica. IPSS was performed as an appropriate means of providing more information about the side of the ACTH source. Immediately after the procedure, the woman experienced double vision, caused by a complete sixth nerve palsy of the left eye, together with a contralateral hemiparesis. From a clinical point of view, abducens nerve palsy and contralateral hemiparesis corresponded to Raymond-Cestan syndrome. I report a case of Raymond-Cestan syndrome, following petrosal sinus sampling in a female patient with ACTH-dependent Cushing's syndrome. To the best of our knowledge, this complication associated with petrosal sinus sampling has only been described in one previous case report. Although IPSS may provide essential information in the investigation of Cushing's syndrome, the possibility of very occasional but devastating complications should be remembered.
Abducens Nerve Diseases ; Adrenocorticotropic Hormone ; Adult ; Brain Stem ; Buffaloes ; Cushing Syndrome ; Diplopia ; Female ; Hemorrhage ; Humans ; Magnetic Resonance Imaging ; Paresis ; Petrosal Sinus Sampling* ; Sella Turcica ; Subarachnoid Hemorrhage

OBJECTIVE: We analyzed the clinical and endocrinological results of the transsphenoidal microsurgery for ACTH secreting pituitary adenomas. MATERIALS AND METHODS: From October 1995 to August 2000, 18 patients underwent transsphenoidal microsurgery for Cushing's disease. We analyzed the surgical results of 17 patients, one patient who was previously operated from other hospital was excluded. Age of the patients were 18 to 61 years old(mean 37.7), male to female ratio was 1: 3.3, and follow-up period was 3 to 50 months(mean 20.3). The selection of candidates for transsphenoidal exploration was based on endocrinologic criteria. Magnetic resonance imaging was the preferred radiologic test. Selective inferior petrosal sinus sampling of adrenocorticotropic hormone futher refined the diagnosis when endocrinologic and radiologic procedures were not definitive. RESULTS: Results of the preoperative endocrinological test were: level of serum ACTH 29.4 to 225ng/dL(mean 93.88ng/dL); serum cortisol 11.9 to 47.5ng/dL(mean 27.49ng/dL); 24-hour urine free cortisol 235 to 1019ng/day(mean 571.0ng/day). Inferior petrosal sinus sampling for ACTH was performed in 11 patients and all were confirmed by Cushing's disease and we could predict the laterality of the tumor in 9 of 11 patients. We performed transsphenoidal selective adenomectomy in 5 patients, adenomectomy and subtotal hypophysectomy in 2 patients, adenomectomy and partial hypophysectomy in 9 patients, and in the remaining one patient, hemihypophysectomy followed by total hypophysectomy due to remission failure. Fifteen of 17 patients(88.2%) showed endocrinological remission. Glucocorticoid replacement therapy was performed in all the patients who showed remission for 1 to 24 months(mean 5.9 months), and 6 patients received steroid over 6 months. CONCLUSION: We conclude that the direct demonstration of a tumor in the pituitary gland by MRI is the most important and definitive diagnostic tool and the location of a mass should be confirmed with increased level of ACTH by the inferior petrosal sinus sampling. Transsphenoidal microsurgery is effective treatment modality for Cushing's disease and the immediate postoperative evaluation of the surgical resection of the tumor is very important. The patients should show hypocortisolism, decreased, subnormal serum ACTH and cortisol levels and 24-hours urine free cortisol. We performed 18 transsphenoidal microsurgery for Cushing's disease in 17 patients and 15 patients(88.2%) showed endocrinological remission.
ACTH-Secreting Pituitary Adenoma ; Adrenocorticotropic Hormone ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hypophysectomy ; Magnetic Resonance Imaging ; Male ; Microsurgery* ; Petrosal Sinus Sampling ; Pituitary Gland